• Journal of Chest Surgery
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• v.30 no.2
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• pp.164-171
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• 1997

Heart transplantation is now accepted as a definitive therapeutic modality in patients with terminal hear failure. The first successful heart transplantation in humans was done in 1967 and the first case in Korea was performed in november, 1992. Since the first case in 1992, more than 50 cases have been performed in Korea. A total of 20 patients underwent orthotopic heart transplantation since November, 1992 in Asan Medicla Center. The purpose of this study is to evaluate the early results and the follow-up course of 20 cases of heart transplantation done in Asan Medical Center. The average age of 20 patients was 39.9$\pm$11.8 years old(20~58). The mean follow-up duration was 14.4$\pm$11.2 months(1~41). All patients are alive till now. The blood type was identical in 14 and compatible in 6 patients. ihe original heart disease was dilated cardiomyopathy in 16, valvular heart disease in 2, ischemic cardiomyopathy in 1, and giant cell myocarditis in 1 patient. HLA cross matching for recipient and donor was done in 18 cases and the results were negative for T-cell and B-cell in 16 patients, pos tive for warm B-cell in 2 patients. Among 6 loci of A, B, and DR, one locus was matched in 8 cases, 2 loci in 5 cases, and 3 loci matched in 1 case. The number of acute allograft rejection averaged 2.8$\pm$0.5 (0~6) per case and the number of acute allograft rejection requiring treatment averaged 1.0$\pm$0.9 (1~3) per case. The time interval from operation to the first acute rejection requiring treatment was 35.5$\pm$20.4 days (5~60). Acute humoral rejection was suspected strongly in 1 case and was successfully treated. The left ventricular ejection fraction measured by echocardiography and/or MUGA scan was dramatically increased from 17.5$\pm$6.8 (9~32)% to 58.9$\pm$2.0 (55~62)% after heart transplantation. Temporary pacing was needed in 5 patients over 24 hours but normal sinus rhythm appeared within 7 days in all cases. One patient has been taken permanent pacemaker implantation due to complete AV block appearing 140 days after heart transplantaion. One patient had cyclosporine-associated n urotoxicity during the immediate postoperative period and was recovered after 27 hours. The heart transplantation of Asan Medical Center is on a developing stage but the early result is comparable to that of well established centers in other countries, even though the long-term follow-up result must be reevaluated. We can conclude that the heart transplantion is a promising therapeutic option in patients with terminal heart failure.

• Tuberculosis and Respiratory Diseases
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• v.46 no.5
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• pp.674-684
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• 1999

Background : It has been generally known that the incidence of lung cancer is higher in the patients with idopathic pumonary fibrosis (IPF) than those in general population. The reported incidence was variable from 4.8 to 43.2%. There were controversies on the most frequent cell type (squamous cell carcinoma vs. adenocarcinoma) and no study was done about the real concordance of cancer and the fibrotic lesion. And the pulmonary fibrosis may influence not only the development of cancer but also the treatment and prognosis of the cancer, but there was no report on that point. Method : Total 63 patients ($66.8{\pm}7.8$ year, M : F=61 : 2) were diagnosed as IPF combined with lung cancer (IFF-CA) at Asan Medical Center. A retrospective analysis was done about the risk factors of the lung cancer, pulmonary function test, the site of cancer(especially the relationship of the cancer with the fibrotic lesion), the histologic types, and the stage of cancer. The histologic types were compared with those of 2,660 patients with lung cancer who were diagnosed at the same institute for the same period. The effect of IPF on the treatment of the cancer was evaluated with the survival time after the detection of lung cancer. Results : The lung cancer was found in 63(22.9%) out of 281 patients with IPF. But in most of them(45 patients), lung cancer was detected at the same time with IPF and only in 18 patients, the cancer was diagnosed during the follow-up($25.2{\pm}17.7$ months) of IPF. So in our study, 6.7% of patients with IPF developed lung cancer during the course of the disease. The age ($66.8{\pm}7.84$ vs. $63.4{\pm}11.1$ years), percentage of smoker (88.9 vs. 67.2%), and the male gender (96.8 vs. 67.6%) were significantly higher in IPF-CA compared with lone IPF (p<0.05). The odds ratio of smoking was 4.7 compared with non smoking IPF controls. The lung cancer was located more frequently in the upper lobe and 55.5% was in the periphery of lung. The cancer was developed in the fibrotic lesion in 23 patients (35.9%), and in the majority of the patients, the cancer was separated from the fibrosis. The cell type of the lung cancer in IPF-CA was squamous cell carcinoma 34.9%, adenocarcinoma 30.2%, small cell carcinoma 19.0%, large cell undifferenciated carcinoma 6.3%, and others 9.5%. No significant difference in the distribution of histologic type of the lung cancer was found between IPF-CA and lone lung cancer. There was no significant difference in demographic features, cell types, location and the stage of the cancer between the group with concurrent IPF-CA and the group with cancer diagnosed during the follow up of IPF. There was a tendency (but statistically not significant : p=0.081) of higher incidence of adenocarcinoma among the cancers developed in the fibrotic area(43.5%) (F-CA) than in the cancers in non-fibrotic area (22.5%) (NF-CA). The prognosis of the patients with F-CA was poor (median survival : 4 months) compared with the patients with NF-CA (7 months, p=0.013), partly because the prevalence of severe IPF (the extent of fibrosis in HRCT 50%) was higher in F-CA group. Conclusion : These data suggest that the lung cancer in the patients with IPF has similar features to the ordinary lung cancer.

• Journal of Chest Surgery
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• v.41 no.1
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• pp.1-11
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• 2008

Background: The Damus-Kaye-Stansel (DKS) procedure is a proximal MPA-ascending aorta anastomosis used to relieve systemic ventricular outflow tract obstructions (SVOTO) and pulmonary hypertension. The purpose of this study was to review the indications and outcomes of the DKS procedure, including the DKS pathway and semilunar valve function. Material and Method: A retrospective review of 28 patients who underwent a DKS procedure between May 1994 and April 2006 was performed. The median age at operation was 5.3 months ($13\;days{\sim}38.1\;months$) and body weight was 5.0 kg ($2.9{\sim}13.5\;kg$). Preoperative pressure gradients were $25.3{\pm}15.7\;mmHg$ ($10{\sim}60\;mmHg$). Eighteen patients underwent a preliminary pulmonary artery banding as an initial palliation. Preoperative main diagnoses were double outlet right ventricle in 9 patients, double inlet left ventricle with ventriculoarterial discordance in 6,. another functional univentricular heart in 5, Criss-cross heart in 4, complete atrioventricular septal defect in 3, and hypoplastic left heart variant in 1. DKS techniques included end-to-side anastomosis with patch augmentation in 14 patients, classical end-to-side anastomosis in 6, Lamberti method (double-barrel) in 3, and others in 5. The bidirectional cavopulmonary shunt and Fontan procedure were concomitantly performed in 6 and 2 patients, respectively. Result: There were 4 hospital deaths (14.3%), and 3 late deaths (12.5%) with a follow-up duration of $62.7{\pm}38.9$ months ($3.3{\sim}128.1$ months). Kaplan-Meier estimated actuarial survival was $71.9%{\pm}9.3%$ at 10 years. Multivariate analysis showed right ventricle type single ventricle (hazard ratio=13.960, p=0.004) and the DKS procedure as initial operation (hazard ratio=6.767, p=0.042) as significant mortality risk factors. Four patients underwent staged biventricular repair and 13 received Fontan completion. No SVOTO was detected after the procedure by either cardiac catheterization or echocardiography except in one patient. There was no semiulnar valve regurgitation (>Gr II) or semilunar valve-related reoperation, but one patient (3.6%) who underwent classical end-to-side anastomosis needed reoperation for pulmonary artery stenosis caused by compression of the enlarged DKS pathway. The freedom from reoperation for the DKS pathway and semilunar valve was 87.5% at 10 years after operation. Conclusion: The DKS procedure can improve the management of SVOTO, and facilitate the selected patients who are high risk for biventricular repair just after birth to undergo successful staged biventricular repair. Preliminary pulmonary artery banding is a safe and effective procedure that improves the likelihood of successful DKS by decreasing pulmonary vascular resistance. The long-term outcome of the DKS procedure for semilunar valve function, DKS pathway, and relief of SVOTO is satisfactory.

• Journal of Chest Surgery
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• v.38 no.8 s.253
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• pp.523-528
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• 2005

Background: Investigation of the change of ventricular function and mitral regurgitation after surgical repair of patient with anomalous origin of left coronary artery from the pulmonary artery (ALCAPA) is key issue for the better surgical outcome. Material and Method: From April 1986 to July 2002, 12 patients presented with ALCAPA. The median age at repair was 4 months. Surgical methods included left coronary artery transfer to the aorta (10), Takeuchi procedure (1), saphenous vein free graft bypass (1). Mitral valve was repaired in 1. Result: There were 2 hospital death $(16.7\%)$. The mean follow-up period was $7.1\pm4.1$ years (range, 7 months to 13 years). Four patients required postoperative circulatory assist for $2.2\pm1.1$ days and one needed left ventricular assist device (LVAD) for 1day. Postoperative echocardiography demonstrated significant improvements in mean fractional shortening $(33.4\pm9.1\%\;vs\;17.7\pm9.6\%,\;n=10,\;p<0.05);$ left ventricular end diastolic dimension $(33.4\pm7.3\;mm\;vs\;44.8\pm7.0\;mm,\;n=10,\;p<0.05)$ and systolic dimension $(22.2\pm7.5\;mm\;vs\;33.4\pm7.9\;mm,\;n=10,\;p<0.05)$. Severities of mitral regurgitation decreased in all survivors at 1st and 4th year follow-up echocardiography. There were 2 reoperation due to residual MR and right ventricular outflow obstruction (Takeuchi case). Conclusion: Anatomic repair of anomalous left coronary artery from the pulmonary artery offered an excellent surgical results, especially in terms of the recovery of left ventricle function and mitral regurgitation. However, preoperative indications for mitral procedure is to be evaluated.

• Journal of Chest Surgery
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• v.35 no.12
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• pp.862-870
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• 2002

The surgical results of the patients with single ventricle(SV) associated with total anomalous pulmonary venous connection(TAPVC) has been reported with high mortality and morbidity due to their morphologic and hemodynamic complexity. A retrospective review was undertaken to report the outcome of the first-stage palliative surgery in our institution and to determine the factors influencing early death. Material and Method: Between January 1987 and June 2002, 39 patients with SV and TAPVC underwent surgical intervention with or without TAPVC repair. Age at operation ranged from 1day to 10.7months (median age, 2.4month), and 29 patients were male. Preoperative diagnosis included 20 right-dominant SV, 15 SV with endocardial cushion defect, 3 left-dominant SV, and 1 tricuspid atresia. The pulmonary venous connection was supracardiac in 22, cardiac in 5, infracardiac in 11, and mixed in 1, Obstructed TAPVC was present in 11. First-stage palliative surgery was performed in 37. Repair of TAPVC, either alone or in association with other procedures, was performed during the initial operation in 31. Univariate and multivariate analyses were performed to analyze the risk factors influencing the operative death. Result: A mean follow-up period of survivors was 34.3 $\pm$ 43.0(0.53 ~ 146.2)months. Overall early operative mortality was 43.6%(17/39). The causes were low cardiac output in 8, failure of weaning from cardiopulmonary bypass in 3, sepsis in 2, pulmonary hypertensive crisis in 1, pulmonary edema in 1, pneumonia in 1, and postoperative arrhythmia in 1. Risk factors influencing early death in univariate analysis were body weight, surgical intervention in neonate, obstructive TAPVC, preoperative conditions including metabolic acidosis, and need for inotropic support, TAPVC repair in initial operation, operative time, and cardiopulmonary bypass(CPB) time. In multivariable analysis, body weight, age at initial operation, surgical intervention in neonate, preoperative conditions including metabolic acidosis, need for inotropic support and CPB time were the risk factors. Conclusion: In this study, we demonstrated that the patients with SV and TAPVC had high perioperative mortality. Preoperative poor condition, young age, the length of operative and CPB time, the presence of obstructive TAPVC had been proven to be the risk factors. This fact suggests that the avoidance of unnecessarily additional procedures may improve the surgical outcomes of the first-stage palliative surgery. However further observation and collection of the data is mandatory to determine the ideal surgical strategy.

• Clinical and Experimental Pediatrics
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• v.48 no.2
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• pp.186-190
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• 2005

Purpose : The purpose of this study was to evaluate the responses and toxicities of risk-adapted chemotherapy in pediatric intracranial germ cell tumors(IC-GCT). Methods : Fourteen patients who were diagnosed as IC-GCT from October 2002 to December 2003 received chemotherapy as an initial treatment modality. The low risk(LR) group was defined as follows : Pure germinoma and normal AFP level. Beta-hCG level 50 mIU/mL or less. The others belonged to the high risk(HR) group. Chemotherapy was composed of cisplatin, cyclophosphamide, etoposide and vincristine. Double doses of cisplatin and cyclophosphamide was used in HR patients. Results : Pathologic confirmation was done in all but one. Median age at diagnosis was 11.6 yr (1.2-18.7 yr), and nine patients belonged to the HR group. Tumor markers were normalized after chemotherapy in all patients whose tumor markers had been elevated. Four LR patients(80 percent) and seven HR patients(77.8 percent) showed complete response(CR) at the end of chemotherapy. An additional two of the three patients with partial response(PR) achieved CR after radiation therapy (RT), and the remaining one relapsed before RT. Four LR and all HR patients experienced infectious episodes that required hospitalization. Four of the nine HR patients(44.4 percent) suffered from tinnitus, three of whom developed sensorineural hearing loss. All but one are surviving, event-free, with a median follow-up of 13.9 mo(8.1-22.3 mo). Conclusion : Risk-adapted cisplatin-based chemotherapy was effective even in HR patients, but regimen modification seems to be necessary to avoid an unacceptably high toxicity rate.

• Radiation Oncology Journal
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• v.21 no.2
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• pp.149-157
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• 2003

Purpose: The purpose of this study was to determine if there were prognostic differences between BRCA related and BRCA non-related Korean patients with early-onset breast carcinomas. Materials and Methods: Sixty women who had developed breast cancers before the age of 40, and who were treated at the Soonchunhyang University Hospital, were studied independently of their family histories. The age range was 18 to 40 with a median of 34.5 years. Lymphocyte specimens from peripheral blood were studied for the heterozygous mutations of BRCA1 and BRCA2 using direct sequencing methods. Immunohistochemistry was peformed on the paraffin-embedded tissue blocks that were available. Results: Eleven deleterious mutations (18.3%, 6 in BRCA1 and 5 in BRCA2) and 7 missense mutations of unknown significance (11.7%), were found among the 60 patients. More than half of the mutation were novel, and were not reported in the database. Most of the BRCA-associated patients had no history of breast cancer. No treatment related failures were observed in the BRCA carriers, with the exception of one patient that had experienced a new primary tumor of the contralateral breast. The seven year relapse free survival rate were 50 and 79% In the BRCA carrier and BRCA negative patients, respectively. Although the expression of estrogen and progesterone receptors were less common, and histological features more aggressive, in the BRCA associated tumors, the outcome of the patients with BRCA mutations was not poorer than that on the patients without deleterious mutations. Conclusion.: Despite the BRCA mutation carriers having adverse prognostic features, the recurrence rate was relatively lower than that in the BRCA non-carrying Korean patients wi4h early-onset breast carcinomas. In addition, although the prevalence of the BRCA mutation in Korean patients was higher than that in white patients, the penetrance of the cancer seemed to be relatively low in Korean women carrying BRCA mutations. A large population based study of the BRCA mutation, with a long-term follow-up of the study patients will be required to confirm these results.

• Tuberculosis and Respiratory Diseases
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• v.41 no.3
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• pp.277-288
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• 1994

Objectives : Multiple lung cancers and/or precancerous lesions can be developed because many bronchi are exposed to carcinogens simultaneously according to the concept of "Field Cancerization". We had performed a careful bronchoscopic examination and analysed the patients of double bronchial lesions who received the separate pathologic evaluation. Methods : We studied 21 patients of double bronchial lesions among 1855 patients of bronchoscopic examination from April 1990 to December 1993 in Korea Cancer Center Hospital. We classified the patients into three groups(double malignancies of different histology, double malignancies of same histology, and combination of malignant and benign lesions) and analysed the histologic type, location, radiologic findings, and clinical parameters. Results : Among 21 patients, six patients had double malignancies of different histology, eight had double malignancies of same histology, and seven had combination of malignant and benign lesions. Out of 14 double malignant cases, 11 cases are considered as synchronous multiple primary lung cancers. Combination of squamous cell carcinomas was found in 5 cases, combination of small cell carcinoma and squamous cell carcinoma was found in 4 cases. Combination of adenocarcinoma and squamous cell carcinoma and combination of squamous cell carcinoma and poorly differentiated carcinoma were found in 1 case respectively. All patients of synchtonous multiple primary lung cancers were male and had long smoking history(average 40 pack years). Among 21 cases of double bronchial lesions, only one lesion could be detected by prebronchoscopic radiologic examination including chest CT in 15 cases. Conclusions : The presence of double bronchial lesions including multiple primary lung cancers and the limitation of radiologic examination to detect early bronchial lesions encourage us to examine the whole bronchi carefully and to perform pathologic evaluations.

• Tuberculosis and Respiratory Diseases
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• v.46 no.5
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• pp.685-696
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• 1999

Background : Idiopathic pulmonary fibrosis (IPF) is a diffuse inflammatory and fibrosing process that occurs within the interstitium and alveolus of the lung with invariably poor prognosis. The major problem in management of IPF results from the variable rate of disease progression and the difficulties in predicting the response to therapy. The purpose of this retrospective study was to evaluate the short-term efficacy of steroid and immunosuppressive therapy for IPF and to identify the pre-treatment determinants of favorable response. Method : Twenty patients of IPF were included. Diagnosis of IPF was proven by thoracoscopic lung biopsy and they were presumed to have active progressive disease. The baseline evaluation in these patients included clinical history, pulmonary function test, bronchoalveolar lavage (BAL), and chest high resolution computed tomography (HRCT). Fourteen patients received oral prednisolone treatment with initial dose of 1mg/kg/day for 8 to 12 weeks and then tapering to low-dose prednisolone (0.25mg/kg/day). Six patients who previously had experienced significant side effects to steroid received 2mg/kg/day of oral cyclophosphamide with or without low-dose prednisolone. Follow-up evaluation was performed after 6 months of therapy. If patients met more than one of followings, they were considered to be responders : (1) improvement of more than one grade in dyspnea index, (2) improvement in FVC or TLC more than 10% or improvement in DLco more than 20% (3) decreased extent of disease in chest HRCT findings. Result : One patient died of extrapulmonary cause after 3 month of therapy, and another patient gave up any further medical therapy due to side effect of steroid. Eventually medical records of 18 patients were analyzed. Nine of 18 patients were classified into responders and the other nine patients into nonresponders. The histopathologic diagnosis of the responders were all nonspecific interstitial pneumonia (NSIP) and that of nonresponders were all usual interstitial pneumonia (UIP) (p<0.001). The other significant differences between the two groups were female predominance (p<0.01), smoking history (p<0.001), severe grade of dyspnea (p<0.05), lymphocytosis in BAL fluid ($23.8{\pm}16.3%$ vs $7.8{\pm}3.6%$, p<0.05), and less honeycombing in chest HRCT findings (0% vs $9.2{\pm}2.3%$, p<0.001). Conclusion : Our results suggest that patients with histopathologic diagnosis of NSIP or lymphocytosis in BAL fluid are more likely to respond to steroid or immunosuppressive therapy. Clinical results in large numbers of IPF patients will be required to identify the independent variables.